Mitral valve replacement in a patient with sickle cell disease using perioperative exchange transfusion

Sickle cell disease is a genetic hemoglobinopathy in which a significant number of red blood cells carry hemoglobin-S as opposed to normal red blood cells that contain hemoglobin-A. Under certain conditions such as hypoxia, acidosis, and hypothermia, the red blood cells containing hemoglobin-S will sickle, leading to occlusion of the microvasculature. As such, patients with sickle cell disease present unique challenges during heart surgery using cardiopulmonary bypass (CPB). After conducting a literature review, we discovered that the exact hemoglobin-S level for conducting cardiac surgery with CPB is not known. However, a hemoglobin-S level < 30% is considered safe for conducting CPB. The following case report will discuss these challenges and present a patient with sickle cell disease undergoing a mitral valve repair. Management of this patient involved exchange transfusions both preoperatively and intraoperatively.